STUDIES ON THE OXYGEN-TRANSPORT IN THE CLINICAL-ASSOCIATION BETWEEN HB-S AND HB-C

The whole blood oxygen affinity of a Negro carrier of SC disease was f ound to be characterized by some right-shifted p50 and clearly increas ed Bohr effect, whereas the isolated and purified Hb-S and Hb-C exhibi ted slight deficiencies mainly of the Bohr effect. The right-shifted p 50 from whole blood can be easily explained by the mild anemia with a parallel increase of 2,3-diphosphoglycerate (DPG), whereas the functio nal discrepancies between whole blood function and that of the purifie d Hb-S and C could be due, at least in part, to the presence in vivo o f consistent amounts of hybrid Hb tetramers of the type alpha alpha be ta S beta C. Unfortunately, the mechanism promoting the formation (or dissolution) of hybrids are fundamentally unknown; so, either their pr esence and functional properties are very difficult to be explored.